Exergame training might offer a novel treatment approach even in largely nonambulatory subjects with multisystemic degenerative spinocerebellar ataxia.

Effective treatments for patients with degenerative spinocerebellar ataxia are scarce. It has recently been shown that intensive coordinative training based on either physiotherapy or exergames (= whole-body controlled videogames; might improve degenerative ataxia, but its effectiveness is still disputed. This situation is even more complicated for degenerative ataxia subjects in advanced disease stages and with high multisystemic disease load. Here, underlying neurodegeneration has progressed to many irreversible states and includes many additional extra-cerebellar systems, making functional plasticity and therapeutic success much less likely. Moreover, intervention outcome assessment in subjects unable to walk freely is more delicate. Correspondingly, nonambulatory ataxia subjects in advanced disease stages are currently often excluded from treatment trials, thus leaving them without prospects of access to novel treatments.

The investigators here hypothesized that exergame training might offer a novel treatment approach even in largely nonambulatory subjects with multisystemic degenerative spinocerebellar ataxia. Using a rater-blinded, intraindividual control study design, the investigators show that an individualized exergame training strategy, tailored to individuals' disease stage, improves postural control and ataxia-specific control functions even in advanced disease.

Inclusion Criteria:

• Advanced spinocerebellar disease
• Advanced impairments in gait and stance, defined as Scale for Assessment and Rating of Ataxia (SARA) subscore gait ≥3 and stance ≥3, but still able to sit without support >10 seconds, as defined by SARA subscore sitting <3
• Age between 5 and 30 years

Exclusion Criteria:

• Any signs of inflammatory, vascular, malformation, or tumor cerebrospinal (CNS) disease
• Visual loss, hearing disturbances, mental retardation and predominant non-ataxia movement disorders (e.g. predominant spasticity, chorea, parkinsonism)